A Long-term Follow-up of Young Adults With Idiopathic Clubfoot: Does Foot Morphology Relate to Pain?

Background: Individuals with clubfoot, treated in infancy with either the Ponseti method or comprehensive clubfoot release, often encounter pain as adults. Multiple studies have characterized residual deformity after Ponseti or surgical correction using physical exam, radiographs and pedobarography; however, the relationship between residual foot deformity and pain is not well defined. The purpose of the current study was 2-fold: (1) to evaluate the relationship between foot morphology and pain for young adults treated as infants for idiopathic clubfoot and (2) to describe and compare pedobarographic measures and outcome measures of pain and morphology among surgically treated, Ponseti treated, and typically developing feet.

Infection profile in children and adolescents with bone marrow failures treated with allogeneic hematopoietic stem cell transplantation.

Background: The objective of the study was to analyze the profile of infections in children with BMF following alloHCT.

Methods: Data of 169 consecutive children with inherited and acquired BMF treated with alloHCT between 2012 and 2017 in Polish pediatric transplant departments were analyzed in registry-based retrospective study, with respect to the type of infection, and clinical outcome.

Results: At least 1 infection was diagnosed in 107/169 patients (60.

Folate Receptor α-Modified Nanoparticles for Targeting of the Central Nervous System.

Effective and timely delivery of therapeutic agents from the systemic circulation to the central nervous system (CNS) is often precluded by the blood-brain barrier (BBB) and the blood-cerebrospinal fluid barrier (BCSFB). A new pathway of folate uptake mediated by folate receptor alpha (FRα, molecular weight of 28.29 kg mol) occurring in various epithelial cells of the CNS (e.

Relationship between burnout and patient safety attitudes in pediatric nurses in a hospital in Turkey.

Aim: Burnout can affect nurses' patient safety-related attitudes due to adverse conditions it creates in nurses and can lead to medical errors. The purpose of this research is to determine the relationship between the patient safety attitudes and pediatric nurses' burnout.

Method: Observational, descriptive and cross-sectional study, carried out between January and July 2016 at Ege University Children's Hospital in İzmir (Turkey).

Genetic, clinical and biochemical characterization of a large cohort of patients with hyaline fibromatosis syndrome.

Background: Hyaline fibromatosis syndrome (HFS) is a rare clinical condition in which bi-allelic variants in ANTXR2 are associated with extracellular hyaline deposits. It manifests as multiple skin nodules, patchy hyperpigmentation, joint contractures and severe pain with movement. HFS shows some clinical overlap to Farber disease (FD), a recessive lysosomal storage disorder.

Multidimensional Developments and Free-Play Movement Tracking in 30- to 36-Month-Old Toddlers With Autism Spectrum Disorder Who Were Full Term.

Background: Few studies have investigated multidimensional developments and free-play movement performance in toddlers with an early diagnosis of autism spectrum disorder (ASD).

Objective: This study compared cognitive, motor, and behavioral developments and free-play movement performance in toddlers with ASD who were full term (FT-ASD), toddlers who were full term and are typically developing (FT-TD), and toddlers who were born preterm and had a very low birth weight (VLBW-PT).

Design: This was a prospective cross-sectional study.

Glomerular Expression of Some Profibrotic Factors in Progressive Childhood Lupus Nephritis.

Background: Lupus nephritis (LN) is a major cause of mortality and morbidity in both adult and pediatric patients. However, studies regarding pathogenesis and predictors of renal outcomes in childhood LN are limited. Transforming growth factor-β1 (TGF-β1) and Connective tissue growth factor (CTGF) have an important role in proliferative and fibrotic changes in many renal diseases.

Primary Failure of the Arteriovenous Fistula in Patients with Chronic Kidney Disease Stage 4/5.

Background: An Arteriovenous fistula (AVF) is a creation of the natural blood vessels. It is a "gate of life" for the patients on hemodialysis.

Aim: The study aimed to analyze the predictors for primary failure of AVF such as gender, age, number and location of AVF, and primary renal disease in patients with chronic kidney disease (CKD) stage 4/5.

Menetrier's disease (protein-losing gastropathy) in a child with acute lymphoblastic leukemia.

A 3-year-old boy with high-risk precursor-B ALL presented with abdominal pain, vomiting, and hypoalbuminemia just before his second scheduled course of high-dose methotrexate in interim maintenance. Examination was significant for epigastric tenderness and periorbital edema. Abdominal imaging revealed a circumferential thickening of the stomach with an increased mucosal enhancement and a mild circumferential thickening of segments of small bowel loops.

Early vocal contact and music in the NICU: new insights into preventive interventions.

It is now clearly established that the environment and the sensory stimuli, particularly during the perinatal period, have an impact on infant's development. During the last trimester of gestation, activity-dependent plasticity shapes the fetal brain, and prematurity has been shown to alter the typical developmental trajectories. In this delicate period, preventive interventions aiming at modulating these developmental trajectories through activity-inducing interventions are currently underway to be tested.

Diagnosis and management of Non-IgE gastrointestinal allergies in breastfed infants-An EAACI Position Paper.

It is well-established that food proteins, such as egg, soya, cow's milk and wheat, are detectable in breastmilk for many hours or days after ingestion. Exposure to these proteins is important to the process of developing tolerance but can also sometimes elicit IgE-mediated and non-IgE-mediated allergic symptoms in breastfed infants. Non-IgE-mediated allergy, outside of food protein-induced allergic proctocolitis and eosinophilic oesophagitis, is not well understood, leading to variations in the diagnosis and management thereof.

Diagnostic Performance Analysis of the Point-of-Care Bilistick System in Identifying Severe Neonatal Hyperbilirubinemia by a Multi-Country Approach.

Importance: The real prevalence and clinical burden of severe neonatal jaundice are undefined due to difficulties in measuring total serum bilirubin (TSB) outside secondary and tertiary clinical centers.

Objective: To assess the diagnostic performance of the point-of care Bilistick System (BS) in identifying neonatal jaundice patients requiring treatment.

Design: Between April 2015 and November 2016, 1911 neonates, were recruited to participate in the study.

Long-term follow-up and comparison of techniques in radiofrequency ablation of ventricular arrhythmias originating from the aortic cusps (AVATAR Registry).

Introduction: Radiofrequency ablation (RFA) of outflow tract ventricular arrhythmia (VA) that originates from the aortic cusps can be challenging. Data on long‑ term efficacy and safety as well as optimal technique after aortic cusp ablation have not previously been reported.

Objectives: This aim of the study was to determine the short- and long‑ term outcomes after RFA of aortic cusp VA, and to evaluate aortic valve injuries according to echocardiographic screening.

Etanercept treatment for extended oligoarticular juvenile idiopathic arthritis, enthesitis-related arthritis, or psoriatic arthritis: 6-year efficacy and safety data from an open-label trial.

Background: To describe the 6-year safety and efficacy of etanercept (ETN) in children with extended oligoarticular juvenile idiopathic arthritis (eoJIA), enthesitis-related arthritis (ERA), and psoriatic arthritis (PsA) METHODS: Patients who completed the 2-year, open-label, phase III CLinical Study In Pediatric Patients of Etanercept for Treatment of ERA, PsA, and Extended Oligoarthritis (CLIPPER) were allowed to enroll in its 8-year long-term extension (CLIPPER2). Children received ETN at a once-weekly dose of 0.8 mg/kg, up to a maximum dose of 50 mg/week.

Plasma Cell Myeloma Type Posttransplant Lymphoproliferative Disorder in an 18-Month-Old Heart Transplant Recipient: Case Report and Review of the Literature.

Plasma cell myeloma type posttransplant lymphoproliferative disorder (PTLD) is a rare subtype of monomorphic B-cell/plasmacytic-type PTLD. Only 10 cases of monomorphic plasmacytic-type PTLD have been previously reported in pediatric transplant recipients (kidney, liver, small bowel-liver, and heart). We present a case of Epstein-Barr virus positive monomorphic plasma cell myeloma type PTLD that developed 10 months after cardiac transplant in an 18-month-old boy.

Accumulation of free cholesterol and oxidized low-density lipoprotein is associated with portal inflammation and fibrosis in nonalcoholic fatty liver disease.

Background: Macrophages engulf oxidized-LDL (oxLDL) leading to accumulation of cellular cholesterol and formation of foam cells, which is a hallmark of atherosclerosis. Moreover, recent studies showed that accumulation of free cholesterol in macrophages leading to activation of NLRP3 inflammasome and production of interleukin-1β (IL-1β) has been linked to atherosclerosis-associated inflammation. However, it is not clear if cholesterol accumulation is associated with hepatic inflammation and fibrosis in the liver.

Whole-exome sequencing detects mutations in pediatric patients with atypical hemolytic uremic syndrome in Taiwan.

Although atypical hemolytic uremic syndrome (aHUS) is a genetic disorder, molecular defects are detected in only 60% of patients. We aim to dissect the genetic background by whole exome sequence and the clinical characteristics of pediatric patients with aHUS. Ten patients (6 male and 4 female) with mean age 5.

The overlooked left ventricle in persistent pulmonary hypertension of the newborn.

Persistent pulmonary hypertension of the newborn (PPHN) is a rapidly increasing condition among neonates. It represents failure of adaptation of pulmonary circulation to the extrauterine environment causing severe hypoxemia in affected newborns. Few data have weighed the relationship of ventricular dysfunction in the context of PPHN and the outcome in involved patients.

Total cerebrovascular blood flow and whole brain perfusion in children sedated using propofol with or without ketamine at induction: An investigation with 2D-Cine PC and ASL.

Background: Multiple sedation regimes may be used to facilitate pediatric MRI scans. These regimes might affect cerebral blood flow and hemodynamics to varying degrees, particularly in children who may be vulnerable to anesthetic side effects.

Purpose: To compare the effects of propofol monosedation solely (Pm group) vs.